Idiopathic Pulmonary Fibrosis

Definition

Idiopathic pulmonary fibrosis (IPF) is a chronic disease. It causes inflammation and fibrosis (scarring) of tissue in the lungs. It occurs most often in people aged 50-70.

There are various types with similar symptoms, but different response to treatment and outcomes.

Causes

Idiopathic means the cause is not known.

Researchers think that IPF is an exaggerated and uncontrolled inflammatory response. This produces the scar tissue. What starts the cycle is not known. Over time, scarring surrounds the thin walled air sacs in the lungs. This makes the tissue thicker and stiffer. As a result, breathing becomes difficult. The lungs gradually lose their ability to pass oxygen to the rest of the body.

Exercise test
on treadmill or stationary bicycle—to measure how well the lungs and heart work during physical activity

Bronchoalveolar lavage—fluid is put into the airways and then removed to study the cells and check for signs of inflammation

Lung biopsy
—a small sample of lung tissue is removed and studied; usually required to confirm a diagnosis of IPF

Treatment

There is no known cure. The goal of treatment is to improve symptoms and slow the disease process. This is done by reducing inflammation and scarring. The tissue that is already scarred cannot be returned to normal.

Medication

Medication is the main form of treatment. It does not work for some people. Options may include:

Combination of prednisone to reduce inflammation and cytoxan or azathioprine to reduce the body’s immune response

To slow the progression of the fibrosis your doctor may recommend:

High dose n-acetylcysteine

Pirfenidone

Interferon-gamma 1b

Coumadin

Etanercept

Bosentan

Imatinib

Sildenafil

Pirfenidone

Colchicine

Methotrexate

Penicillamine

Cyclosporine

Gastroesophageal Reflux Disease

GERD will need to be treated. This most often involves the use of medication and lifestyle changes.

Support Care

Some people may need to receive oxygen. This will help them breathe.

A pulmonary rehab program may also improve lung function.

A healthy lifestyle may also help slow the disease. This includes:

Not smoking

Healthy diet

Regular exercise

Rest

Lung transplantation
may be considered for people with advanced IPF who do not respond to other treatment.

Prevention

There is no proven way to prevent IPF. However, avoiding smoking and wearing masks for work in some occupations may help.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.